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DBT and ACADM
DBT
ACADM
Description
dihydrolipoamide branched chain transacylase E2
acyl-CoA dehydrogenase medium chain
Image
GO Annotations
Cellular Component
Mitochondrion
Mitochondrial Matrix
Cytosol
Microtubule Cytoskeleton
Mitochondrial Nucleoid
Branched-chain Alpha-ketoacid Dehydrogenase Complex
Nucleus
Cytoplasm
Mitochondrion
Mitochondrial Matrix
Axon
Mitochondrial Membrane
Molecular Function
Protein Binding
Transferase Activity
Acyltransferase Activity
Acyltransferase Activity, Transferring Groups Other Than Amino-acyl Groups
Ubiquitin Protein Ligase Binding
Dihydrolipoamide Branched Chain Acyltransferase Activity
Acyl-CoA Dehydrogenase Activity
Oxidoreductase Activity
Oxidoreductase Activity, Acting On The CH-CH Group Of Donors
Identical Protein Binding
Flavin Adenine Dinucleotide Binding
Medium-chain Fatty Acyl-CoA Dehydrogenase Activity
Biological Process
Branched-chain Amino Acid Catabolic Process
Branched-chain Alpha-keto Acid Decarboxylation To Branched-chain Acyl-CoA
Liver Development
Glycogen Biosynthetic Process
Organic Acid Metabolic Process
Regulation Of Gluconeogenesis
Lipid Metabolic Process
Fatty Acid Metabolic Process
Fatty Acid Beta-oxidation
Heart Development
Response To Cold
Carnitine Metabolic Process
Post-embryonic Development
Carnitine Metabolic Process, CoA-linked
Fatty Acid Beta-oxidation Using Acyl-CoA Dehydrogenase
Response To Starvation
Carnitine Biosynthetic Process
Medium-chain Fatty Acid Metabolic Process
Medium-chain Fatty Acid Catabolic Process
Cardiac Muscle Cell Differentiation
Pathways
Branched-chain amino acid catabolism
RHOH GTPase cycle
Mitochondrial protein degradation
Protein lipoylation
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Loss-of-function mutations in DBT cause MSUD2
Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD
Loss-of-function mutations in DLD cause MSUD3/DLDD
Branched-chain ketoacid dehydrogenase kinase deficiency
H139Hfs13* PPM1K causes a mild variant of MSUD
PPARA activates gene expression
mitochondrial fatty acid beta-oxidation of unsaturated fatty acids
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
Beta oxidation of octanoyl-CoA to hexanoyl-CoA
Drugs
Octanoyl-Coenzyme A
Flavin adenine dinucleotide
3-thiaoctanoyl-CoA
Diseases
Maple syrup urine disease (MSUD)
Disorders of fatty-acid oxidation, including: Medium-chain (MC) acyl-CoA dehydrogenase (AD) deficiency (MCADD); Short-chain AD deficiency (SCADD) ; Short-branched-chain AD deficiency (SBCADD) ; Very long-chain AD deficiency (VLCADD) ; Long-chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHADD) ; Trifunctional protein deficiency (TFP); Carnitine palmitoyltransferase deficiency (CPT); Carnitine-acylcarnitine translocase deficiency (CACT); Systemic primary carnitine deficiency (CDSP)
GWAS
Lung cancer in ever smokers (
28604730
)
Daytime sleep phenotypes (
27126917
)
Glycine levels (
30837465
31070104
)
Metabolic traits (
21886157
)
Metabolite levels (
20037589
)
Serum metabolite concentrations in chronic kidney disease (
29545352
)
Serum metabolite levels (
23093944
33031748
)
Serum metabolite ratios in chronic kidney disease (
29545352
)
Urinary metabolite levels in chronic kidney disease (
31959995
)
Urinary metabolite modules (eigenmetabolites) in chronic kidney disease (
31959995
)
Interacting Genes
1 interacting genes:
OGT
5 interacting genes:
CALM1
CDC42
DYNC1I1
PANK1
SUMO4
Entrez ID
1629
34
HPRD ID
02010
08447
Ensembl ID
ENSG00000137992
ENSG00000117054
Uniprot IDs
A0A7P0T9W1
P11182
A0A0S2Z366
B4DJE7
B7Z9I1
P11310
Q5T4U5
PDB IDs
1K8M
1K8O
1ZWV
2COO
3RNM
1EGC
1EGD
1EGE
1T9G
2A1T
4P13
8SGP
Enriched GO Terms of Interacting Partners
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Regulation Of Gluconeogenesis
Regulation Of Glucose Metabolic Process
Mitochondrial Membrane
Protein N-acetylglucosaminyltransferase Complex
Negative Regulation Of Non-canonical Inflammasome Complex Assembly
Regulation Of Small Molecule Metabolic Process
Carnitine Metabolic Process, CoA-linked
Medium-chain Fatty Acyl-CoA Dehydrogenase Activity
Medium-chain Fatty Acid Catabolic Process
Carnitine Biosynthetic Process
Protein O-acetylglucosaminyltransferase Activity
Positive Regulation Of Transcription From RNA Polymerase II Promoter By Glucose
Non-canonical Inflammasome Complex Assembly
Positive Regulation Of Transcription By Glucose
Regulation Of Transcription From RNA Polymerase II Promoter By Glucose
Response To Nutrient Levels
Carbon Catabolite Regulation Of Transcription
Medium-chain Fatty Acid Metabolic Process
Regulation Of Transcription By Glucose
Carnitine Metabolic Process
Fatty Acid Beta-oxidation Using Acyl-CoA Dehydrogenase
Acyl-CoA Dehydrogenase Activity
NSL Complex
Amino-acid Betaine Metabolic Process
Glycogen Biosynthetic Process
Regulation Of Neurotransmitter Receptor Localization To Postsynaptic Specialization Membrane
Acetylglucosaminyltransferase Activity
Polysaccharide Biosynthetic Process
Oxidoreductase Activity, Acting On The CH-CH Group Of Donors
Sin3-type Complex
Histone Acetyltransferase Complex
Negative Regulation Of Stem Cell Population Maintenance
Regulation Of Protein Localization To Synapse
Regulation Of Receptor Localization To Synapse
Regulation Of Necroptotic Process
Regulation Of Rac Protein Signal Transduction
Cardiac Muscle Cell Differentiation
Phosphatidylinositol-3,4,5-trisphosphate Binding
Cellular Response To Nutrient
Regulation Of Programmed Necrotic Cell Death
Response To Cold
Pyroptotic Inflammatory Response
Cardiocyte Differentiation
Negative Regulation Of Translational Initiation
Negative Regulation Of Proteasomal Ubiquitin-dependent Protein Catabolic Process
Positive Regulation Of Translational Initiation
Positive Regulation Of Stem Cell Population Maintenance
Mitochondrion
Glycogen Metabolic Process
Energy Reserve Metabolic Process
Intracellular Signaling Cassette
Small GTPase-mediated Signal Transduction
Angiotensin Type I Receptor Activity
Bradykinin Receptor Binding
Substantia Nigra Development
Negative Regulation Of High Voltage-gated Calcium Channel Activity
Neural Nucleus Development
Regulation Of Cytokinesis
Negative Regulation Of Ryanodine-sensitive Calcium-release Channel Activity
Cellular Response To Type II Interferon
Cytoplasmic Ribonucleoprotein Granule
GBD Domain Binding
Storage Vacuole
Organelle Transport Along Microtubule
Dihydrolipoamide Branched Chain Acyltransferase Activity
Recombination Nodule
Phytanic Acid Metabolic Process
Pantothenate Kinase Activity
Angiotensin Type II Receptor Activity
Phospholipase C-activating Angiotensin-activated Signaling Pathway
Renin-angiotensin Regulation Of Aldosterone Production
Organelle Localization
Response To Type II Interferon
Nucleotide Binding
Apolipoprotein A-I Receptor Binding
Endothelin Receptor Signaling Pathway Involved In Heart Process
Neuropilin Signaling Pathway
Branched-chain Alpha-keto Acid Decarboxylation To Branched-chain Acyl-CoA
Branched-chain Alpha-ketoacid Dehydrogenase Complex
Methyl-branched Fatty Acid Metabolic Process
Long-chain Fatty Acid Import Into Peroxisome
Acetyl-CoA Binding
SUMO Ligase Complex
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Tagcloud (Intersection)
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